免疫原
Synthesized peptide derived from human ACADL AA range: 258-308
特异性
This antibody detects endogenous levels of ACADL at Human/Mouse/Rat
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（Background）
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008],
功能
catalytic activity:Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.,cofactor:FAD.,disease:Impaired ACADL activity results in non-ketotic hypoglycemia, hypotonia, muscle weakness and episodes of cardiorespiratory arrest associated with fasting [MIM:201460].,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homotetramer.,
Fields
>>Fatty acid degradation;>>Metabolic pathways;>>Fatty acid metabolism;>>PPAR signaling pathway
细胞定位
Mitochondrion matrix .
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